Health and social care needs of people living with dementia: a qualitative study of dementia support in the Victorian region of Gippsland, Australia.

INTRODUCTION: There is a lack of information about the experiences of people living with dementia and their carers, especially in rural and regional areas. Understanding these experiences helps to identify gaps and unmet needs within the health system and improve quality of care and outcomes for people living with dementia. The aim of this study was to improve our knowledge of dementia support needs. This included access to health and social care services and supports for people living with dementia and those who provide informal or formal support to someone living with dementia. METHODS: Interviews were conducted with 26 participants from the Gippsland region of Victoria, Australia with knowledge of dementia care. Purposive sampling engaged people with lived experience, carers/family members and health professionals delivering dementia care and social services. Discussions centred around participants' experiences of support services, the diagnosis process and what they thought was needed to improve the services and supports offered. Thematic analysis of the data was undertaken using the framework method. RESULTS: The interview data indicated that the needs of many people living with dementia and their carers were not currently being met. The themes were limited access to services and supports, including primary and specialist care, often impacted by lack of knowledge of care options, difficulty navigating the system and funding models as a barrier, leading to delays in getting a diagnosis and accessing specialist services; lack of holistic care to enable people living with dementia to 'live well'; and stigma impacted by a lack of knowledge of dementia among professionals and in the community. Relationship-centred care was described as a way to improve the lives of people living with dementia. CONCLUSION: Key areas for improvement include increasing community awareness of dementia and available local services, more support to obtain an early dementia diagnosis, increased help to navigate the system, especially immediately after diagnosis, and easier access to appropriate home support services when they are needed. Other recommendations include person-centred care across settings - supported by funding models, more education and communication skills training for health professionals and care staff - and greater support for and increased recognition of carers.

Difficult case of a rare form of familial Alzheimer's disease with PSEN1 P117L mutation.

Less than 10% of Alzheimer's disease (AD) cases are familial. Presenilin-1 (PSEN1) mutations are the most frequent aetiology and may be associated to atypical neurological manifestations. We report the case of a 27-year-old right-handed man, ensuing with mild cognitive impairment, motor discoordination and axial myoclonus after a parachute accident. At age 32 he was referred to our neurology clinic, presenting cognitive impairment, cerebellar syndrome, axial myoclonus and hypomimia, without other signs of parkinsonism. Because of absence of family history, he was worked up along the line of spinal ataxic disorders. Later, he developed pseudobulbar affect, cognitive deterioration, right upper limb paresis and spastic paraparesis. Subsequent investigation identified a PSEN1 P117L mutation and the diagnosis of autosomal dominant AD was made. This case illustrates the diagnostic challenge imposed by atypical presentation of de novo PSEN1 mutation, leading to unnecessary investigation. Genetic study might be essential for defining the diagnosis.

Parkinson's Disease and development of levodopa induced motor complications: Influence of baseline features and first medical approach.

BACKGROUND: The introduction of levodopa in clinical practice represents a hallmark in the treatment of the neurodegenerative disease, Parkinson's Disease. However, levodopa induced motor complications, namely dyskinesias and motor fluctuations, develop in the majority of Parkinson's Disease patients. OBJECTIVE: to identify which Parkinson's Disease's, patient's and therapeutics' initial features are more associated with dyskinesias or motor fluctuations development. METHODS: Patients with diagnosed Parkinson's Disease attending neurology outpatient clinic at Centro Hospitalar São João were selected. For this observational study, data was retrospectively collected from patient's clinical records. A survival analysis model with univariate and multivariate regression analysis was used. RESULTS: 87 patients with a mean of 72 ± 9.7 years were included. After a median follow-up of 6 (range 1-17) years, 35.6% patients developed dyskinesias; and with a median of 5 (range 1-16) years, 32.2% developed motor fluctuations. After multivariate analysis, the akinesia/rigidity subtype was found to have a higher risk of dyskinesias and motor fluctuations development. Age of onset ≤50 years was associated with motor fluctuations development. CONCLUSION: In conclusion, our results suggest that Parkinson's Disease patients' initial characteristics, such as subtype or age of onset, are independently associated with the development of motor complications.

Isolated III cranial nerve palsy: a Hodgkin's lymphoma?

A 69-year-old woman developed ptosis and diplopia due to an isolated pupil-involving left oculomotor nerve palsy. General examination was unremarkable. Initial workup showed a mild increase in cerebrospinal fluid proteins. Imaging studies were remarkable for a left oculomotor nerve enhancement in brain MRI and hyperfixation along the nerve's pathway in full body single-photon emission CT. Assuming the possible diagnosis of neurosarcoidosis, the patient was started on high-dose methylprednisolone. Three months later she developed pancytopenia. A bone marrow biopsy was performed and histopathology revealed infiltration by Hodgkin's lymphoma. Adriamycin, bleomycin, vinblastine, dacarbazine protocol chemotherapy was started and full haematological remission obtained after four cycles, despite mild oculomotor nerve palsy persisted. Isolated oculomotor palsy as the first presenting manifestation of a lymphoma is rare and alternative differential diagnosis must be considered in the absence of other lymphoma manifestations. In this case as with many rare initial manifestations of common diseases watchful waiting was crucial to the correct diagnosis and treatment strategy.

Exercise in the community for people with multiple sclerosis--a follow-up of people with minimal gait impairment.

BACKGROUND: Although there are many studies evaluating exercise interventions, few studies have evaluated the effect at follow-up. OBJECTIVES: This paper presents follow-up data for participants who completed the exercise interventions in a large randomised controlled trial. METHODS: One hundred twenty-one people with multiple sclerosis (MS) with minimal gait impairment who completed 10 weeks of community-based exercise interventions were evaluated by a blinded assessor 12 weeks after the intervention. The primary outcome measure was the Multiple Sclerosis Impact Scale-29 version 2 (MSIS-29,v2) physical component. Other outcomes were the MSIS-29 psychological component, the Modified Fatigue Impact Scale (MFIS) and the 6-minute walk test (6MWT) distance. RESULTS: The positive effect on the physical impact of MS was not maintained from baseline to follow-up (-1.6, 95% CI -0.8, 4.0, p=0.189). The psychological impact and the impact of fatigue remained significantly improved (-3.5, 95% CI -6.1, -1.0, p = 0.006 and -4.68, 95% CI -6.9, -2.5, p < 0.001, respectively). There was no time effect for the 6MWT (f = 1.76, p = 0.179) although the trend suggests reversal of the benefits gained from the physiotherapist (PT)- and fitness instructor (FI)-led intervention. CONCLUSION: The maintained benefit on the psychological impact of MS and fatigue may have important personal and socioeconomic consequences; however, it is important to find ways to maintain the physical benefits of exercise over the long term.

Reversible parkinsonism due to a large intracranial tumour.

A 77-year-old woman presented with progressively worsening apathy, depression, urinary incontinence and slowness of movement for the past 1 year. Asymmetric akinetic-rigid parkinsonism and mild left-sided hyper-reflexia were seen on examination. No ocular movement impairment, cerebellar or sensory signs were noticed. Routine laboratory testing was normal. Brain imaging revealed a large frontal tumour which was subsequently excised and pathologically confirmed as a meningioma. Marked clinical improvement was documented 3 months after surgery, and persistent clinical and imaging remission have been confirmed annually for the following 3 years. There have been some reports of parkinsonism associated with intracranial tumours. Although this is probably an uncommon situation, it is potentially treatable, and symptoms might even remit completely following successful management. Parkinson's disease is a common cause of parkinsonism, but alternative aetiologies should be suspected whenever atypical findings are demonstrated by clinical history or examination.

Stretching the limbs? Tonic spasms in multiple sclerosis.

A 23-year-old man with a clinically isolated syndrome (right optic neuritis) diagnosed 6 months before, presented with recurrent, brief, painful, stereotyped, involuntary posturing movements of the left upper limb. The neurological examination was otherwise unremarkable (except for right optic atrophy). Intravenous methylprednisolone was initiated; the paroxysms persisted and worsened 7 days later, as the left lower limb and hemiface became affected. A video-EEG showed no epileptiform activity despite the movements. Brain MRI revealed new lesions affecting the right pyramidal tract, contralateral to the clinical manifestations. Valproate was prescribed and the paroxysms were completely resolved 5 days later. Tonic spasms are classically, although infrequently, seen in multiple sclerosis, and may clinically resemble primary paroxysmal dyskinesias or even focal motor epileptic seizures.

The role of breast MRI in the investigation of anti-Yo positive paraneoplastic cerebellar degeneration.

Paraneoplastic neurological syndromes are a group of rare and heterogeneous disorders complicating cancer through immune-mediated mechanisms. They typically arise before the diagnosis of malignancy, thus constituting its first clinical manifestation. A thorough search for the underlying tumour is necessary, as adequate tumour management is essential for both neurological prognosis and overall survival. The authors present the case of a 43 year-old woman who presented with a subacute cerebellar syndrome associated with the paraneoplastic anti-Yo antibody. Although paraneoplastic aetiology was immediately suspected, the diagnosis of the underlying tumour was not straightforward, as is often the case. This case report highlights the importance of directing tumour search for the most probable anatomic locations, according to patient demographics and risk factors as well as the type of onconeural antibodies present, and also the need to use the most sensitive diagnostic modalities appropriate for each target organ.

Getting the balance right: a randomised controlled trial of physiotherapy and Exercise Interventions for ambulatory people with multiple sclerosis.

BACKGROUND: People with Multiple Sclerosis have a life long need for physiotherapy and exercise interventions due to the progressive nature of the disease and their greater risk of the complications of inactivity. The Multiple Sclerosis Society of Ireland run physiotherapy, yoga and exercise classes for their members, however there is little evidence to suggest which form of physical activity optimises outcome for people with the many and varied impairments associated with MS. METHODS AND DESIGN: This is a multi-centre, single blind, block randomised, controlled trial. Participants will be recruited via the ten regional offices of MS Ireland. Telephone screening will establish eligibility and stratification according to the mobility section of the Guys Neurological Disability Scale. Once a block of people of the same strand in the same geographical region have given consent, participants will be randomised. Strand A will concern individuals with MS who walk independently or use one stick to walk outside. Participants will be randomised to yoga, physiotherapy led exercise class, fitness instructor led exercise class or to a control group who don't change their exercise habits.Strand B will concern individuals with MS who walk with bilateral support or a rollator, they may use a wheelchair for longer distance outdoors. Participants will be randomised to 1:1 Physiotherapist led intervention, group intervention led by Physiotherapist, group yoga intervention or a control group who don't change their exercise habits. Participants will be assessed by physiotherapist who is blind to the group allocation at week 1, week 12 (following 10 weeks intervention or control), and at 12 week follow up. The primary outcome measure for both strands is the Multiple Sclerosis Impact Scale. Secondary outcomes are Modified Fatigue Impact Scale, 6 Minute Walk test, and muscle strength measured with hand held dynamometry. Strand B will also use Berg Balance Test and the Modified Ashworth Scale. Confounding variables such as sensation, coordination, proprioception, range of motion and other impairments will be recorded at initial assessment. DISCUSSION: Data analysis will analyse change in each group, and the differences between groups. Sub group analysis may be performed if sufficient numbers are recruited. TRIAL REGISTRATION: ISRCTN77610415.

Structure and ultrafast dynamics of white-light-emitting CdSe nanocrystals.

White-light emission from ultrasmall CdSe nanocrystals offers an alternative approach to the realization of solid-state lighting as an appealing technology for consumers. Unfortunately, their extremely small size limits the feasibility of traditional methods for nanocrystal characterization. This paper reports the first images of their structure, which were obtained using aberration-corrected atomic number contrast scanning transmission electron microscopy (Z-STEM). With subangstrom resolution, Z-STEM is one of the few available methods that can be used to directly image the nanocrystal's structure. The initial images suggest that they are crystalline and approximately four lattice planes in diameter. In addition to the structure, for the first time, the exciton dynamics were measured at different wavelengths of the white-light spectrum using ultrafast fluorescence upconversion spectroscopy. The data suggest that a myriad of trap states are responsible for the broad-spectrum emission. It is hoped that the information presented here will provide a foundation for the future development and improvement of white-light-emitting nanocrystals.

Effects of surface passivation on the exciton dynamics of CdSe nanocrystals as observed by ultrafast fluorescence upconversion spectroscopy.

The exciton dynamics of CdSe nanocrystals are intimately linked to the surface morphology. Photo-oxidation of the selenium surfaces of the nanocrystal leads to an increase in radiative decay efficiency from both the band edge and deep trap emission states. The addition of the primary amine hexadecylamine curtails nonradiative excitonic decay attributed to the dangling surface selenium orbitals by passivation of those trap sites by the methylene protons on the amine, leading to enhanced band edge emission and the absence of deep trap emission. Furthermore, CdSeZnSe core/shell nanocrystals are not immune from contributions from surface states because of the alignment of the band structures of the core and shell materials.